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DOI: 10.1055/s-0045-1806544
The IgG4-related cholangitis – distinguishing from other benign biliary strictures: clinical features, imaging and response to treatment
Authors
Aims IgG4-related cholangitis (IRC) is uncommon systemic fibroinflammatory disorder and challenging in diagnosis which can involve multiple secretory organs in human body. There have multiple modalities approach for diagnosis including imaging, laboratory test and histopathology. The IRC can mimic either benign biliary stricture (BBS) or malignant biliary stricture (MBS). The IRC have a dramatically response to systemic steroid which is a hallmark feature. However, misdiagnosis in this condition may lead to inappropriate medical and invalidating surgery. We aimed to compare these baseline characteristics, laboratory test, imaging and response of treatment between the IRC and other BBS groups [1] [2] [3] [4] [5] [6] [7] [8].
Methods All cases of definite diagnosis of benign biliary stricture (BBS) patients between January 2013 and January 2023 were reviewed. Endoscopic diagnostic and treatment was performed; data pertaining to serum bilirubin level, serum IgG4, stent type and response to treatment were obtained. The baseline characteristic and treatment of biliary stricture and stent indwelling time were compared between groups.
Results A total of benign biliary stricture 158 patients (IRC, n=19; other BBS, n=139) underwent endoscopic diagnosis and treatment. The mean age was 59 year-old and 62% were male. The presence of comorbid disease, and initial laboratory results were not significantly different between the two groups. The clinical presentation of obstructive jaundice and concomitant with autoimmune pancreatitis had higher in IRC than other BBS (73.7% vs. 29.7%, p<0.001) and (52.6% vs. 0%, p<0.001) respectively. As expected, the serum IgG4 level had significant higher in IRC than in BBS (5.384 g/L vs. 0.838 g/L, p<0.001). Moreover, the stricture length of IRC had significant longer 23 mm compared with 7 mm in other BBS. The diagnosis of IRC can achieve the complete response by medication without need prolonged endoscopic stenting or surgery. The crucial factors associated with diagnosis of IRC were male (OR 3.71, 95% CI 1.03-13.32, p=0.045), presenting with obstructive jaundice (OR 6.62, 95% CI 2.24-19.59, p=0.001), symmetrical bile duct thickening (OR 5.00, 95% CI 1.82-13.69, p=0.002) and long stricture length>15 mm (OR 5.72, 95% CI 2.02-16.19, p=0.001) and tissue lymphoplasmacytic infiltration (OR 39.96, 95% CI 7.46-214.10, p<0.001)
Conclusions IRC is distinguishing BBS which have specific clinical features, imaging, characteristics of stricture mimic with malignant feature. The advantage of the serum IgG4 and tissue stain for IgG4 positive cell lead to accurate diagnosis. Recognize of IRC in the low prevalence area guided to additional specific work up and treatment to avoiding unnecessary surgery.
Publication History
Article published online:
27 March 2025
© 2025. European Society of Gastrointestinal Endoscopy. All rights reserved.
Georg Thieme Verlag KG
Oswald-Hesse-Straße 50, 70469 Stuttgart, Germany
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